The Multi-Detector CT Findings of Giant Abdominal Lymphangiectasis Mimicking a Mesenteric Cystic Mass in a Patient with Midgut Volvulus

Case Report

The Multi-Detector CT findings of giant abdominal lymphangiectasis mimicking a mesenteric cystic mass in a patient with midgut volvulus

Midgut Volvuluslu Bir Hastada Mezenterik Kistik Kitleyi Taklit Eden Dev Abdominal Lenfanjiyektazinin ÇKBT Bulguları

Mecit Kantarci1, Selim Doganay2, Serpil Kurtcan1, Cemal Gundogdu3, Akgun Oral4, Berrin Demir1

1Department of Radiology, Medical Faculty, Atatürk University, Erzurum, Turkey 2Department of Radiology, Develi Government Hospital, Kayseri, Turkey 3Department of Pathology, Medical Faculty, Atatürk University, Erzurum, Turkey 4Department of Pediatric Surgery, Numune Hospital, Erzurum, Turkey

Correspondence to: Mecit Kantarci, 200 Evler Mah. 14. Sok No 5, Dadaskent, Erzurum, Turkey. Phone: +90.442.3273802 (home), +90.442.2361212-1521 (work), fax: +90.442.2361301, e-mail: [email protected]

Abstract Özet

Our purpose is to show the Multi-Detector CT (MDCT) findings of an Bizim bu yazıdaki amacımız, midgut volvulusun eşlik ettiği mezen- intra-abdominal giant cystic lesion with midgut volvulus and to share terik kisti taklit eden dev lenfanjiyektazi olgusunun çok kesitli BT our experience with giant lymphangiectasis mimicking a mesenteric (ÇKBT) bulgularını sunmaktır. Malrotasyon çocuklarda erişkinlere cyst or a cystic mass. The pathological evaluation indicated that the göre daha sık görülebilir. Hastalarda kronik ve tekrarlayan volvu- cyst contained abdominal lymphatic material. Malrotation is usually luslar venöz konjesyona ve lenfatik göllenmelere neden olur. Post detected in infants and children, but rarely in adults. In patients with operatif patolojik değerlendirmede kistik yapının abdominal lenfa- chronic and recurrent volvulus, chronic venous congestion with lym- tik materyal ihtiva ettiği belirlendi. Lenfatik drenaj obstrüksiyonları phatic engorgement may occur. Interference with lymphatic drainage lenfanjiyomaya ya da şilöz mezenterik kistlere neden olabilir. Bizim may result in formation of a lymphangioma or a chylous mesenteric vakamızda da intestinal lenfatik torsiyon mezenterik kisti taklit eden cyst. In our case, because of the intestinal lymphatic torsion, there kistik lezyona neden olmuştu. Literatür bilgilerimize göre bizim va- was a cystic lesion mimicking a mesenteric cyst or a cystic mass. To kamız midgut volvulus ve geniş mezenterik lenfanjiyektazinin ÇKBT our knowledge, this is the first case of midgut volvulus and large bulgularının sunulduğu ilk vakadır. mesenteric lymphangiectasis to be depicted by MDCT.

Keywords: Intestinal lymphangiectasis , MDCT, Midgut volvulus Anahtar Kelimeler: ÇKBT, İntestinal lenfanjiyektazi, Midgut volvulus

The Eurasian Journal of Medicine 94 Kantarci et al.

Introduction Systems, Tokyo, Japan). A MDCT angiography examination was performed with low-radiation-dose techniques and with the fol- lowing technical parameters: a detector collimation of 16x0.5, a pitch of 1.75, a reconstruction interval of 1 mm, a slice thickness alrotation is a non-specific term that comprises a variety of 1.25 mm, a table speed of 14 mm/sec, a gantry rotation time M of anomalies in intestinal rotation and fixation [1]. It is of 0.5 sec, and settings of 30 mAs, and 80 kVp. The patient re- usually detected in infants and children, but rarely in ceived nonionic intravenous (IV) contrast material (320 mg/ mL) adults. The most important complication of malrotation is midgut at a dose of 2 mL per kilogram of body weight. The scan was volvulus, which can result in bowel necrosis [2]. In midgut volvu- initiated 12-15 sec after the start of the IV contrast material injec- lus, the lymphatic ducts are also torsioned with the mesenteric tion. Three-dimensional (3D) volume-rendered (VR) images were vein, the artery, and intestinal loops. In this report, we present obtained from axial images at a separate workstation to display the MDCT findings of a case with significantly dilated, tortuous vascular and osseous structures. The abdominal MDCT showed a abdominal lymphatic ducts mimicking a cystic mass and accom- twist of the mesenteric vessels, suggestive of a midgut volvulus, panying midgut volvulus. and there was a multicystic mass with smooth contours in the right lower quadrant of the abdomen (Fig. 2a). The superior me- Case Report senteric vein (SMV) was positioned abnormally to the left of the superior mesenteric artery (SMA), and the SMA was encircled counterclockwise by the SMV and bowel (Fig. 2b). The MDCT scan provided no further information about the cystic lesion. Be- A 7-year-old boy was admitted to the emergency depart- cause the complaints of the patient significantly regressed within ment with abdominal pain, reduced appetite, nausea, and vomit- hours, the patient was enrolled into follow-up. On the control US ing. The physical examination revealed severe peritoneal irritation obtained the next day, the lesion appeared to have a reduced on deep palpation of the right lower quadrant. The white blood size (8 cm) and to have switched position downwards. After 48 cell count was 17.100 /mL3. Serum biochemistry tests were hours, the complaints of the patient significantly increased, so within normal limits. Plain films of the abdomen revealed disten- emergent surgery was performed. tion of the stomach and duodenum. Abdominal gray-scale sonog- Intraoperatively, our patient was found to have malrotation raphy showed an 11-cm heterogeneous, semi-solid cystic mass in with midgut volvulus and a large cystic mass. The mesentery was the right lower quadrant of the abdomen (Fig. 1). In addition, a detorsioned 540 degrees in a counter-clockwise fashion, includ- whirlpool sign, indicating midgut volvulus, was demonstrated on ing the mesenteric vein, mesenteric artery, and lymphatic ducts. the emergent abdominal color Doppler ultrasound (US). A MDCT The lymphatic ducts were severely dilated. After derotation of scan was performed to acquire more information for a differen- the midgut, the small bowel was placed at the right side of the tial diagnosis of the lesion. An abdominal CT examination includ- abdomen, and the colon was placed at the left side. All the in- ing sagittal and coronal reconstructed images was performed testines were viable, and no bowel resection was required. Pr- with a 16-detector-row CT scanner (Aquillon; Toshiba Medical eoperatively, on the other hand, the cystic mass in the lower right quadrant was found to have a soft constitution, smooth contours, and a slightly reduced size. The cystic mass was left in its place after a biopsy sample was obtained. The pathological evaluation of the cystic mass indicated that it contained abdominal lymphatic material (Fig. 3). On the control US obtained after one month, the cystic lesion appeared to have regressed and then completely resolved.

Discussion

MDCT can be very useful in the diagnosis of midgut volvulus. MDCT is a fast and technically easy way to fully assess patients with midgut volvulus. The characteristic appearance of a twist- ed mesentery, collapsed small bowel loops, and mesenteric fat wrapping around the SMA is pathognomonic and is commonly referred to as the ‘’whirl sign’’ or clockwise whirlpool sign’’ [3-6]. In our case, intestinal obstruction at the duodenojejunal junc- tion, dilated duodenum and stomach, and the whirl sign were depicted on MDCT. In addition, an 11-cm heterogeneous, semi- solid cystic mass in the right lower quadrant of the abdomen was Fig. 1 _ The ultrasound study demonstrates a semi-solid cystic mass that depicted by ultrasonography. In this case, the MDCT scan has heterogenic echogenity and is 11 cm in size.

EAJM: 40, August 2008 95 MDCT Findings of Giant Abdominal Lymphangiectasis

Fig. 2a Fig. 2b

Fig. 2 _ Contrast-enhanced axial MDCT images show a hypodense non-enhanced multicystic mass in the right lower quadrant of the abdomen (a). Contrast- enhanced axial MDCT imaging shows the level of superior mesenteric vessels with characteristic clockwise twisting of the bowel, mesentery, and superior mesenteric vein around the axis of superior mesenteric artery (b).

was performed to obtain further information for a differen- In conclusion, the lymphatic ducts are also torsioned with tial diagnosis of the cystic lesion; however, no further information the mesenteric vein, the artery, and intestinal loops in midgut was obtained. volvulus. Giant lymphangiectasis secondary to lymphatic torsion In older children, the symptoms of malrotation with volvu- may accompany midgut volvulus and mimic a mesenteric cyst lus are usually atypical and vague, resulting in delayed diagnosis or cystic mass. In that case, lymphatic dilatation associated with [7,8]. In patients with chronic and recurrent volvulus, chronic volvulus should be considered during differential diagnosis. venous congestion with lymphatic engorgement may occur [8]. Interference with lymphatic drainage may result in formation of a lymphangioma or a chylous mesenteric cyst [7]. In our case, because of the intestinal lymphatic torsion, there was cystic lesion mimicking a mesenteric cyst or a cystic mass. Mesenteric lymphatic vessels, which are very small, unite to form the intestinal trunk, which enters the cisterna chyli. The intestinal trunk receives the lymph from the stomach, intestine, pancreas, spleen, and liver. The size of the intestinal lymphatic vessels varies markedly depending on whether the patient is fast- ing [9]. Intestinal lymphangiectasis is characterized by tortuosity and marked dilatation of mesenteric lymphatic vessels. Dilatation of the lymphatic vessels and of the cisterna chyli may be observed after surgical ligation of the thoracic duct during gastric or esophageal surgery [10]. Our patient had a large multicystic lesion that accompanied mesenteric torsion and was located in the right lower quadrant. In the light of the above information, we hypothesized that the marked lymphatic dilatation due to mesenteric torsion in our patient was mimicking a mesenteric cyst or cystic mass. After the torsion was corrected through surgery, the control US showed that the cyst disappeared on its own ac- cord, and the biopsy results indicated lymphatic material. These findings are supportive of our hypothesis. To our knowledge, this is the first case of midgut volvulus ac- _ companied by large mesenteric lymphangiectasis to be depicted Fig. 3 At HE x 100 magnification, extremely enlarged lymphatic structures by MDCT. with lumens filled with eosinophilic content and congested vascular structures adjacent to the fibro-adipose tissue are observed.

EAJM: 40, August 2008 96 Kantarci et al.

Conflict interest statement The authors declare that they have no conflict of interest to the publication of this article.

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